Scarring/arrhythmogenic cardiomyopathy

Area of Science:

• Cardiology
• Cardiovascular Imaging
• Genetics

Background:

• Arrhythmogenic cardiomyopathy (ACM) is evolving beyond right ventricular involvement to include left ventricular (LV) and biventricular (BIV) forms.
• Scarring/arrhythmogenic cardiomyopathy (S/ACM) is proposed as an umbrella term emphasizing myocardial scarring from myocyte death, leading to arrhythmias and systolic dysfunction.
• Distinguishing S/ACM from non-scarring heart diseases like dilated cardiomyopathy is crucial for accurate diagnosis and management.

Purpose of the Study:

• To introduce the term 'scarring/arrhythmogenic cardiomyopathy (S/ACM)' to encompass diverse S/ACM phenotypes.
• To highlight the role of the 2020 Padua criteria in improving S/ACM diagnosis through cardiac magnetic resonance (CMR) imaging.
• To discuss the challenges in risk stratification and implantable cardioverter defibrillator (ICD) selection for S/ACM patients.

Main Methods:

• Review of evolving concepts in arrhythmogenic cardiomyopathy.
• Emphasis on myocardial scarring as a key pathological feature.
• Incorporation of cardiac magnetic resonance (CMR) findings in diagnostic criteria (Padua criteria).

Main Results:

• S/ACM is characterized by ventricular myocardium loss and replacement with fibrous or fibro-fatty scar tissue.
• The Padua criteria enhance S/ACM diagnosis by integrating CMR myocardial tissue characterization.
• Risk stratification relies primarily on arrhythmic burden and ventricular dysfunction, with ongoing challenges in ICD patient selection.

Conclusions:

• S/ACM represents a spectrum of conditions unified by myocardial scarring, necessitating refined diagnostic approaches.
• The Padua criteria and CMR imaging are pivotal in advancing S/ACM diagnosis and understanding.
• Effective management requires addressing arrhythmias, heart failure, and optimizing ICD therapy selection.